Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

U. A. Walker; A. Tyndall; L. Czirják; C. Denton; D. Farge-Bancel; O. Kowal-Bielecka; U. Müller-Ladner; C. Bocelli-Tyndall; M. Matucci-Cerinic; Gabriela Riemekasten; Claudia Brückner; Paolo Airó; Mirko Scarsi; Raffaella Scorza; Lorenzo Beretta; Franco Cozzi; Francesco Tiso; M. C. Vonk; F. H.J. Van Den Hoogen; Fredrick M. Wigley; Laura Hummers; Tatjana Nevskaya; Lidia Ananieva; Irene Miniati; Nicoletta Tartaglia; Claudia Lomater; Alexandra Balbir-Gurman; Yolanda Braun-Moscovici; Lisa Maria Bambara; Paola Caramaschi; Gabriele Valentini; Luigia Ruocco; Thomas Krieg; Nicolas Hunzelmann; Cecília Varjú; Patricia E. Carriera; Beatriz Joven; Florenzo Iannone; Giovanni Lapadula; André Kahan; Yannick Allanore; Armando Gabrielli; Michele Imperatore; Agneta Scheja; Frank Wollheim; Nemanja Damjanov; Predrag Ostojic; Petra Saar; Ingo H. Tarner; Ina Kötter; Stefano Bombardieri; Laura Bazzichi; Nicoletta Del Papa; Denise P. Comina; Andrea Lo Monaco; Renato La Corte; Eric Hachulla; David Launay; Oliver Distler; Adrian Ciurea; Stanislaw Sierakowski; Holly Mitchell; Richard M. Silver; Dorota Krasowska; Malgorzata Michalska-Jakubus; Mohammed Tikly; Nazrana Aboo; Margitta Worm; Pascal Klaus; Jozef Rovenský; Olga Lukáčová; Blaz Rozman; Alenka Sipek; Paulo Clemente-Coelho; Yehuda Shoenfeld; Pnina Langewitch; A. P. Da Silva José; M. J. Salvador; Annegret Kuhn; Gunilla Erdmann; Radim Bečvář; Elke Friedl; Winfried Graninger; Valeria Riccieri; Roberto Caporali; Carlomaurizio Montecucco; P. Vlachoyiannopoulos; Meike Distler; Kristian Reich; Maria Majdan; Ewa Wielosz; Simona Rednic; Jacob M. Van Laar; Stefan Heitmann; Andreas Bruckner; Andrea Himsel; Julia Riemann; Rotraud Meyringer; Adelheid Müller; Duska Martinovic; Mislav Radic; Michael Sticherling; Zoltan Szekanecz; Gabriella Szücs; Roberto Giacomelli; Alessandra Marrelli; Bojana Stamenkovic; Aleksandra Stankovic; Martin Aringer; Josef S. Smolen; E. Kucharz; Anna T. Kotulska; Stefania Jablonska; Maria Blasczik; Jae Bum Jun; Carmel Mallia; Bernard Coleiro; Vera Ortiz Santamaria; Ralf Hinrichs; Henrik Nielsen; Roberta Cossutta; Ruxandra Ionescu; Daniela Opris; Kerstin Steinbrink; Boris Grundt; Gianluigi Bajocchi; Štork Jiří; Paloma García De La Peña Lefebvre; Antonio C. Zea Mendoza; Camillo Ribi; Carlo Chizzolini; Margaret Wisłowska; Srdan Novak; Francesco Indiveri; Søren Jacobsen; Per Brown Frandsen; I. Zimmermann Gorska; Jane Tore Gran; Øyvind Midtvedt; Filipa Oliveira Ramos; Ljubinka Damjanovska Rajcevska; Georgi Bozinovski; Dieter Schöffel; Cord Sunderkötter; Markus Böhm; Jadranka Morović-Vergles; Melanie Ivana Čulo; Maurizio Cutolo; Alberto Sulli; Chris T. Derk; Sergio A. Jimenez; Panagiota Siakka; Klaus Søndergaard; Kristian Stengaard-Pedersen; Jean Cabane; Kiet Phong Tiev; Carina Mihai; Roxana Sfrent-Cornateanu; Michael Jendro; Piia Tuvik; Marco Antivalle; Giovanna Randisi; Matthias Seidel; Ricarda Clarenbach; Ismail Simsek; Ayhan Dinc; Murat Inanc; Monica Sinziana Capraru; Dorin Capraru; Inmaculada Bañegil; Jutta Richter; Saad Alhasani; Ivan Földvari; Sandra Pinto; Filipe Brandão; Antonio Juan Mas

doi:10.1136/ard.2006.062901

Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

U. A. Walker^*, A. Tyndall, L. Czirják, C. Denton, D. Farge-Bancel, O. Kowal-Bielecka, U. Müller-Ladner, C. Bocelli-Tyndall, M. Matucci-Cerinic, Gabriela Riemekasten, Claudia Brückner, Paolo Airó, Mirko Scarsi, Raffaella Scorza, Lorenzo Beretta, Franco Cozzi, Francesco Tiso, M. C. Vonk, F. H.J. Van Den Hoogen, Fredrick M. WigleyLaura Hummers, Tatjana Nevskaya, Lidia Ananieva, Irene Miniati, Nicoletta Tartaglia, Claudia Lomater, Alexandra Balbir-Gurman, Yolanda Braun-Moscovici, Lisa Maria Bambara, Paola Caramaschi, Gabriele Valentini, Luigia Ruocco, Thomas Krieg, Nicolas Hunzelmann, Cecília Varjú, Patricia E. Carriera, Beatriz Joven, Florenzo Iannone, Giovanni Lapadula, André Kahan, Yannick Allanore, Armando Gabrielli, Michele Imperatore, Agneta Scheja, Frank Wollheim, Nemanja Damjanov, Predrag Ostojic, Petra Saar, Ingo H. Tarner, Ina Kötter, Stefano Bombardieri, Laura Bazzichi, Nicoletta Del Papa, Denise P. Comina, Andrea Lo Monaco, Renato La Corte, Eric Hachulla, David Launay, Oliver Distler, Adrian Ciurea, Stanislaw Sierakowski, Holly Mitchell, Richard M. Silver, Dorota Krasowska, Malgorzata Michalska-Jakubus, Mohammed Tikly, Nazrana Aboo, Margitta Worm, Pascal Klaus, Jozef Rovenský, Olga Lukáčová, Blaz Rozman, Alenka Sipek, Paulo Clemente-Coelho, Yehuda Shoenfeld, Pnina Langewitch, A. P. Da Silva José, M. J. Salvador, Annegret Kuhn, Gunilla Erdmann, Radim Bečvář, Elke Friedl, Winfried Graninger, Valeria Riccieri, Roberto Caporali, Carlomaurizio Montecucco, P. Vlachoyiannopoulos, Meike Distler, Kristian Reich, Maria Majdan, Ewa Wielosz, Simona Rednic, Jacob M. Van Laar, Stefan Heitmann, Andreas Bruckner, Andrea Himsel, Julia Riemann, Rotraud Meyringer, Adelheid Müller, Duska Martinovic, Mislav Radic, Michael Sticherling, Zoltan Szekanecz, Gabriella Szücs, Roberto Giacomelli, Alessandra Marrelli, Bojana Stamenkovic, Aleksandra Stankovic, Martin Aringer, Josef S. Smolen, E. Kucharz, Anna T. Kotulska, Stefania Jablonska, Maria Blasczik, Jae Bum Jun, Carmel Mallia, Bernard Coleiro, Vera Ortiz Santamaria, Ralf Hinrichs, Henrik Nielsen, Roberta Cossutta, Ruxandra Ionescu, Daniela Opris, Kerstin Steinbrink, Boris Grundt, Gianluigi Bajocchi, Štork Jiří, Paloma García De La Peña Lefebvre, Antonio C. Zea Mendoza, Camillo Ribi, Carlo Chizzolini, Margaret Wisłowska, Srdan Novak, Francesco Indiveri, Søren Jacobsen, Per Brown Frandsen, I. Zimmermann Gorska, Jane Tore Gran, Øyvind Midtvedt, Filipa Oliveira Ramos, Ljubinka Damjanovska Rajcevska, Georgi Bozinovski, Dieter Schöffel, Cord Sunderkötter, Markus Böhm, Jadranka Morović-Vergles, Melanie Ivana Čulo, Maurizio Cutolo, Alberto Sulli, Chris T. Derk, Sergio A. Jimenez, Panagiota Siakka, Klaus Søndergaard, Kristian Stengaard-Pedersen, Jean Cabane, Kiet Phong Tiev, Carina Mihai, Roxana Sfrent-Cornateanu, Michael Jendro, Piia Tuvik, Marco Antivalle, Giovanna Randisi, Matthias Seidel, Ricarda Clarenbach, Ismail Simsek, Ayhan Dinc, Murat Inanc, Monica Sinziana Capraru, Dorin Capraru, Inmaculada Bañegil, Jutta Richter, Saad Alhasani, Ivan Földvari, Sandra Pinto, Filipe Brandão, Antonio Juan Mas

^*Corresponding author for this work

Clinic of Rheumatology

589 Citations (Scopus)

Abstract

Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.

Original language	English
Journal	Annals of the Rheumatic Diseases
Volume	66
Issue number	6
Pages (from-to)	754-763
Number of pages	10
ISSN	0003-4967
DOIs	https://doi.org/10.1136/ard.2006.062901
Publication status	Published - 06.2007

Research Areas and Centers

Academic Focus: Center for Infection and Inflammation Research (ZIEL)

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1136/ard.2006.062901

Cite this

Walker, U. A., Tyndall, A., Czirják, L., Denton, C., Farge-Bancel, D., Kowal-Bielecka, O., Müller-Ladner, U., Bocelli-Tyndall, C., Matucci-Cerinic, M., Riemekasten, G., Brückner, C., Airó, P., Scarsi, M., Scorza, R., Beretta, L., Cozzi, F., Tiso, F., Vonk, M. C., Van Den Hoogen, F. H. J., ... Mas, A. J. (2007). Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database. Annals of the Rheumatic Diseases, 66(6), 754-763. https://doi.org/10.1136/ard.2006.062901

@article{eb2ef920af124ac49a1cfdd1343fe9c1,

title = "Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database",

abstract = "Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.",

author = "Walker, {U. A.} and A. Tyndall and L. Czirj{\'a}k and C. Denton and D. Farge-Bancel and O. Kowal-Bielecka and U. M{\"u}ller-Ladner and C. Bocelli-Tyndall and M. Matucci-Cerinic and Gabriela Riemekasten and Claudia Br{\"u}ckner and Paolo Air{\'o} and Mirko Scarsi and Raffaella Scorza and Lorenzo Beretta and Franco Cozzi and Francesco Tiso and Vonk, {M. C.} and {Van Den Hoogen}, {F. H.J.} and Wigley, {Fredrick M.} and Laura Hummers and Tatjana Nevskaya and Lidia Ananieva and Irene Miniati and Nicoletta Tartaglia and Claudia Lomater and Alexandra Balbir-Gurman and Yolanda Braun-Moscovici and Bambara, {Lisa Maria} and Paola Caramaschi and Gabriele Valentini and Luigia Ruocco and Thomas Krieg and Nicolas Hunzelmann and Cec{\'i}lia Varj{\'u} and Carriera, {Patricia E.} and Beatriz Joven and Florenzo Iannone and Giovanni Lapadula and Andr{\'e} Kahan and Yannick Allanore and Armando Gabrielli and Michele Imperatore and Agneta Scheja and Frank Wollheim and Nemanja Damjanov and Predrag Ostojic and Petra Saar and Tarner, {Ingo H.} and Ina K{\"o}tter and Stefano Bombardieri and Laura Bazzichi and {Del Papa}, Nicoletta and Comina, {Denise P.} and {Lo Monaco}, Andrea and {La Corte}, Renato and Eric Hachulla and David Launay and Oliver Distler and Adrian Ciurea and Stanislaw Sierakowski and Holly Mitchell and Silver, {Richard M.} and Dorota Krasowska and Malgorzata Michalska-Jakubus and Mohammed Tikly and Nazrana Aboo and Margitta Worm and Pascal Klaus and Jozef Rovensk{\'y} and Olga Luk{\'a}{\v c}ov{\'a} and Blaz Rozman and Alenka Sipek and Paulo Clemente-Coelho and Yehuda Shoenfeld and Pnina Langewitch and {Da Silva Jos{\'e}}, {A. P.} and Salvador, {M. J.} and Annegret Kuhn and Gunilla Erdmann and Radim Be{\v c}v{\'a}{\v r} and Elke Friedl and Winfried Graninger and Valeria Riccieri and Roberto Caporali and Carlomaurizio Montecucco and P. Vlachoyiannopoulos and Meike Distler and Kristian Reich and Maria Majdan and Ewa Wielosz and Simona Rednic and {Van Laar}, {Jacob M.} and Stefan Heitmann and Andreas Bruckner and Andrea Himsel and Julia Riemann and Rotraud Meyringer and Adelheid M{\"u}ller and Duska Martinovic and Mislav Radic and Michael Sticherling and Zoltan Szekanecz and Gabriella Sz{\"u}cs and Roberto Giacomelli and Alessandra Marrelli and Bojana Stamenkovic and Aleksandra Stankovic and Martin Aringer and Smolen, {Josef S.} and E. Kucharz and Kotulska, {Anna T.} and Stefania Jablonska and Maria Blasczik and Jun, {Jae Bum} and Carmel Mallia and Bernard Coleiro and Santamaria, {Vera Ortiz} and Ralf Hinrichs and Henrik Nielsen and Roberta Cossutta and Ruxandra Ionescu and Daniela Opris and Kerstin Steinbrink and Boris Grundt and Gianluigi Bajocchi and {\v S}tork Ji{\v r}{\'i} and Lefebvre, {Paloma Garc{\'i}a De La Pe{\~n}a} and {Zea Mendoza}, {Antonio C.} and Camillo Ribi and Carlo Chizzolini and Margaret Wis{\l}owska and Srdan Novak and Francesco Indiveri and S{\o}ren Jacobsen and Frandsen, {Per Brown} and Gorska, {I. Zimmermann} and {Tore Gran}, Jane and {\O}yvind Midtvedt and Ramos, {Filipa Oliveira} and Rajcevska, {Ljubinka Damjanovska} and Georgi Bozinovski and Dieter Sch{\"o}ffel and Cord Sunderk{\"o}tter and Markus B{\"o}hm and Jadranka Morovi{\'c}-Vergles and {\v C}ulo, {Melanie Ivana} and Maurizio Cutolo and Alberto Sulli and Derk, {Chris T.} and Jimenez, {Sergio A.} and Panagiota Siakka and Klaus S{\o}ndergaard and Kristian Stengaard-Pedersen and Jean Cabane and Tiev, {Kiet Phong} and Carina Mihai and Roxana Sfrent-Cornateanu and Michael Jendro and Piia Tuvik and Marco Antivalle and Giovanna Randisi and Matthias Seidel and Ricarda Clarenbach and Ismail Simsek and Ayhan Dinc and Murat Inanc and Capraru, {Monica Sinziana} and Dorin Capraru and Inmaculada Ba{\~n}egil and Jutta Richter and Saad Alhasani and Ivan F{\"o}ldvari and Sandra Pinto and Filipe Brand{\~a}o and Mas, {Antonio Juan}",

year = "2007",

month = jun,

doi = "10.1136/ard.2006.062901",

language = "English",

volume = "66",

pages = "754--763",

journal = "Annals of the Rheumatic Diseases",

issn = "0003-4967",

number = "6",

}

Walker, UA, Tyndall, A, Czirják, L, Denton, C, Farge-Bancel, D, Kowal-Bielecka, O, Müller-Ladner, U, Bocelli-Tyndall, C, Matucci-Cerinic, M, Riemekasten, G, Brückner, C, Airó, P, Scarsi, M, Scorza, R, Beretta, L, Cozzi, F, Tiso, F, Vonk, MC, Van Den Hoogen, FHJ, Wigley, FM, Hummers, L, Nevskaya, T, Ananieva, L, Miniati, I, Tartaglia, N, Lomater, C, Balbir-Gurman, A, Braun-Moscovici, Y, Bambara, LM, Caramaschi, P, Valentini, G, Ruocco, L, Krieg, T, Hunzelmann, N, Varjú, C, Carriera, PE, Joven, B, Iannone, F, Lapadula, G, Kahan, A, Allanore, Y, Gabrielli, A, Imperatore, M, Scheja, A, Wollheim, F, Damjanov, N, Ostojic, P, Saar, P, Tarner, IH, Kötter, I, Bombardieri, S, Bazzichi, L, Del Papa, N, Comina, DP, Lo Monaco, A, La Corte, R, Hachulla, E, Launay, D, Distler, O, Ciurea, A, Sierakowski, S, Mitchell, H, Silver, RM, Krasowska, D, Michalska-Jakubus, M, Tikly, M, Aboo, N, Worm, M, Klaus, P, Rovenský, J, Lukáčová, O, Rozman, B, Sipek, A, Clemente-Coelho, P, Shoenfeld, Y, Langewitch, P, Da Silva José, AP, Salvador, MJ, Kuhn, A, Erdmann, G, Bečvář, R, Friedl, E, Graninger, W, Riccieri, V, Caporali, R, Montecucco, C, Vlachoyiannopoulos, P, Distler, M, Reich, K, Majdan, M, Wielosz, E, Rednic, S, Van Laar, JM, Heitmann, S, Bruckner, A, Himsel, A, Riemann, J, Meyringer, R, Müller, A, Martinovic, D, Radic, M, Sticherling, M, Szekanecz, Z, Szücs, G, Giacomelli, R, Marrelli, A, Stamenkovic, B, Stankovic, A, Aringer, M, Smolen, JS, Kucharz, E, Kotulska, AT, Jablonska, S, Blasczik, M, Jun, JB, Mallia, C, Coleiro, B, Santamaria, VO, Hinrichs, R, Nielsen, H, Cossutta, R, Ionescu, R, Opris, D, Steinbrink, K, Grundt, B, Bajocchi, G, Jiří, Š, Lefebvre, PGDLP, Zea Mendoza, AC, Ribi, C, Chizzolini, C, Wisłowska, M, Novak, S, Indiveri, F, Jacobsen, S, Frandsen, PB, Gorska, IZ, Tore Gran, J, Midtvedt, Ø, Ramos, FO, Rajcevska, LD, Bozinovski, G, Schöffel, D, Sunderkötter, C, Böhm, M, Morović-Vergles, J, Čulo, MI, Cutolo, M, Sulli, A, Derk, CT, Jimenez, SA, Siakka, P, Søndergaard, K, Stengaard-Pedersen, K, Cabane, J, Tiev, KP, Mihai, C, Sfrent-Cornateanu, R, Jendro, M, Tuvik, P, Antivalle, M, Randisi, G, Seidel, M, Clarenbach, R, Simsek, I, Dinc, A, Inanc, M, Capraru, MS, Capraru, D, Bañegil, I, Richter, J, Alhasani, S, Földvari, I, Pinto, S, Brandão, F & Mas, AJ 2007, 'Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database', Annals of the Rheumatic Diseases, vol. 66, no. 6, pp. 754-763. https://doi.org/10.1136/ard.2006.062901

TY - JOUR

T1 - Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

AU - Walker, U. A.

AU - Tyndall, A.

AU - Czirják, L.

AU - Denton, C.

AU - Farge-Bancel, D.

AU - Kowal-Bielecka, O.

AU - Müller-Ladner, U.

AU - Bocelli-Tyndall, C.

AU - Matucci-Cerinic, M.

AU - Riemekasten, Gabriela

AU - Brückner, Claudia

AU - Airó, Paolo

AU - Scarsi, Mirko

AU - Scorza, Raffaella

AU - Beretta, Lorenzo

AU - Cozzi, Franco

AU - Tiso, Francesco

AU - Vonk, M. C.

AU - Van Den Hoogen, F. H.J.

AU - Wigley, Fredrick M.

AU - Hummers, Laura

AU - Nevskaya, Tatjana

AU - Ananieva, Lidia

AU - Miniati, Irene

AU - Tartaglia, Nicoletta

AU - Lomater, Claudia

AU - Balbir-Gurman, Alexandra

AU - Braun-Moscovici, Yolanda

AU - Bambara, Lisa Maria

AU - Caramaschi, Paola

AU - Valentini, Gabriele

AU - Ruocco, Luigia

AU - Krieg, Thomas

AU - Hunzelmann, Nicolas

AU - Varjú, Cecília

AU - Carriera, Patricia E.

AU - Joven, Beatriz

AU - Iannone, Florenzo

AU - Lapadula, Giovanni

AU - Kahan, André

AU - Allanore, Yannick

AU - Gabrielli, Armando

AU - Imperatore, Michele

AU - Scheja, Agneta

AU - Wollheim, Frank

AU - Damjanov, Nemanja

AU - Ostojic, Predrag

AU - Saar, Petra

AU - Tarner, Ingo H.

AU - Kötter, Ina

AU - Bombardieri, Stefano

AU - Bazzichi, Laura

AU - Del Papa, Nicoletta

AU - Comina, Denise P.

AU - Lo Monaco, Andrea

AU - La Corte, Renato

AU - Hachulla, Eric

AU - Launay, David

AU - Distler, Oliver

AU - Ciurea, Adrian

AU - Sierakowski, Stanislaw

AU - Mitchell, Holly

AU - Silver, Richard M.

AU - Krasowska, Dorota

AU - Michalska-Jakubus, Malgorzata

AU - Tikly, Mohammed

AU - Aboo, Nazrana

AU - Worm, Margitta

AU - Klaus, Pascal

AU - Rovenský, Jozef

AU - Lukáčová, Olga

AU - Rozman, Blaz

AU - Sipek, Alenka

AU - Clemente-Coelho, Paulo

AU - Shoenfeld, Yehuda

AU - Langewitch, Pnina

AU - Da Silva José, A. P.

AU - Salvador, M. J.

AU - Kuhn, Annegret

AU - Erdmann, Gunilla

AU - Bečvář, Radim

AU - Friedl, Elke

AU - Graninger, Winfried

AU - Riccieri, Valeria

AU - Caporali, Roberto

AU - Montecucco, Carlomaurizio

AU - Vlachoyiannopoulos, P.

AU - Distler, Meike

AU - Reich, Kristian

AU - Majdan, Maria

AU - Wielosz, Ewa

AU - Rednic, Simona

AU - Van Laar, Jacob M.

AU - Heitmann, Stefan

AU - Bruckner, Andreas

AU - Himsel, Andrea

AU - Riemann, Julia

AU - Meyringer, Rotraud

AU - Müller, Adelheid

AU - Martinovic, Duska

AU - Radic, Mislav

AU - Sticherling, Michael

AU - Szekanecz, Zoltan

AU - Szücs, Gabriella

AU - Giacomelli, Roberto

AU - Marrelli, Alessandra

AU - Stamenkovic, Bojana

AU - Stankovic, Aleksandra

AU - Aringer, Martin

AU - Smolen, Josef S.

AU - Kucharz, E.

AU - Kotulska, Anna T.

AU - Jablonska, Stefania

AU - Blasczik, Maria

AU - Jun, Jae Bum

AU - Mallia, Carmel

AU - Coleiro, Bernard

AU - Santamaria, Vera Ortiz

AU - Hinrichs, Ralf

AU - Nielsen, Henrik

AU - Cossutta, Roberta

AU - Ionescu, Ruxandra

AU - Opris, Daniela

AU - Steinbrink, Kerstin

AU - Grundt, Boris

AU - Bajocchi, Gianluigi

AU - Jiří, Štork

AU - Lefebvre, Paloma García De La Peña

AU - Zea Mendoza, Antonio C.

AU - Ribi, Camillo

AU - Chizzolini, Carlo

AU - Wisłowska, Margaret

AU - Novak, Srdan

AU - Indiveri, Francesco

AU - Jacobsen, Søren

AU - Frandsen, Per Brown

AU - Gorska, I. Zimmermann

AU - Tore Gran, Jane

AU - Midtvedt, Øyvind

AU - Ramos, Filipa Oliveira

AU - Rajcevska, Ljubinka Damjanovska

AU - Bozinovski, Georgi

AU - Schöffel, Dieter

AU - Sunderkötter, Cord

AU - Böhm, Markus

AU - Morović-Vergles, Jadranka

AU - Čulo, Melanie Ivana

AU - Cutolo, Maurizio

AU - Sulli, Alberto

AU - Derk, Chris T.

AU - Jimenez, Sergio A.

AU - Siakka, Panagiota

AU - Søndergaard, Klaus

AU - Stengaard-Pedersen, Kristian

AU - Cabane, Jean

AU - Tiev, Kiet Phong

AU - Mihai, Carina

AU - Sfrent-Cornateanu, Roxana

AU - Jendro, Michael

AU - Tuvik, Piia

AU - Antivalle, Marco

AU - Randisi, Giovanna

AU - Seidel, Matthias

AU - Clarenbach, Ricarda

AU - Simsek, Ismail

AU - Dinc, Ayhan

AU - Inanc, Murat

AU - Capraru, Monica Sinziana

AU - Capraru, Dorin

AU - Bañegil, Inmaculada

AU - Richter, Jutta

AU - Alhasani, Saad

AU - Földvari, Ivan

AU - Pinto, Sandra

AU - Brandão, Filipe

AU - Mas, Antonio Juan

PY - 2007/6

Y1 - 2007/6

N2 - Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.

AB - Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.

UR - http://www.scopus.com/inward/record.url?scp=34248379222&partnerID=8YFLogxK

U2 - 10.1136/ard.2006.062901

DO - 10.1136/ard.2006.062901

M3 - Journal articles

C2 - 17234652

AN - SCOPUS:34248379222

SN - 0003-4967

VL - 66

SP - 754

EP - 763

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

IS - 6

ER -

Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

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